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For patients with amyotrophic lateral sclerosis (ALS), the simple act of breathing is often a tough thing to do. Now, these patients have new hope, as a device has been approved by the U.S. Food and Drug Administration (FDA) that may help patients to breathe more easily without a ventilator.
ALS, also known as Lou Gehrig's disease, is a rapidly progressing, incurable and fatal neuromuscular disease characterized by progressive muscle weakness that results in paralysis. For ALS patients, diaphragm weakness contributes significantly to respiratory insufficiency, and ultimately respiratory failure, which is the most common cause of death for these patients. As the phrenic nerve to the diaphragm muscle fails, patients lose the ability to breathe without ventilator support.
Approximately 30,000 people in the United States live with ALS. More than 5,600 new cases are diagnosed each year, and about 3,300 of those patients might benefit from a diaphragmatic pacing system (DPS).
I recently worked with my colleague, Dr. Patrick Ross, a thoracic surgeon at The Ohio State University Wexner Medical Center to implant the NeuRx DPS device in Mary Pat Murry of London, Ohio. Our integrated ALS clinic and surgical center is the first to offer this new device to patients in Ohio, Kentucky and Indiana. We are one of only 30 integrated, multidisciplinary ALS clinics nationwide currently offering the device.
The device provides electrical stimulation to the nerve that innervates the diaphragm, similar to how a cardiac pacemaker works to stimulate the heart. When the device stimulates the nerve, the diaphragm contracts, and this helps condition the muscle to improve fatigue resistance during normal exertion.
Most ALS patients develop chronic hypoventilation over the course of their disease. Traditionally, we assist them with non-invasive ventilator support. The hope is that the diaphragmatic pacing system will help patients breathe for a longer period without needing a ventilator.
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The DPS treatment is working well for Murry, whose respiratory function initially increased and has now plateaued, instead of continuing to decline. She tells me she feels more rested and has more energy since she has been using the DPS about 10 hours a day.
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In 2014, Ohio State will participate in a national multi-center clinical trial that will try to determine if DPS treatment for patients with ALS and hypoventilation will improve survival or diaphragm function. About 20 Northeast ALS Consortium (NEALS) centers in the United States will participate in the randomized study that will enroll 180 patients. Study participants will be randomly assigned in a 2:1 ratio to DPS or standard-of-care treatment groups.
In an earlier multi-center clinical trial, the device was approved by the FDA under a Humanitarian Device Exemption marketing approval, based on demonstration of safety and probable benefit, including the likelihood that it will help ALS patients live longer and sleep better than the current standard of care. The device is controlled through a four-channel, battery-powered, external pulse generator.
The NeuRx DPS was developed over 20 years through a joint effort of physicians and engineers at several institutions.
ALS is incurable and right now physicians can't stop its progress, but we are optimistic that this will give people with ALS a better quality of life during the course of their disease.
The views expressed are those of the author and do not necessarily reflect the views of the publisher. This version of the article was originally published on LiveScience.
